Pourfour du Petit syndrome associated with a cervical vertebral anomaly.

Pourfour du Petit syndrome is a rare dysautonomic disorder characterized by mydriasis, eyelid retraction, and hyperhidrosis and is caused by irritative stimulation of the sympathetic cervical chain. The authors describe a 45-year-old woman with iris heterochromia, who presented with episodes of ipsilateral mydriasis and hyperhidrosis and was found to have a cervical vertebral anomaly, probably present since birth, as the cause of Pourfour du Petit syndrome.

Altered antioxidant-oxidant status in the aqueous humor and peripheral blood of patients with retinitis pigmentosa.

Retinitis Pigmentosa is a common form of hereditary retinal degeneration constituting the largest Mendelian genetic cause of blindness in the developed world. It has been widely suggested that oxidative stress possibly contributes to its pathogenesis. We measured the levels of total antioxidant capacity, free nitrotyrosine, thiobarbituric acid reactive substances (TBARS) formation, extracellular superoxide dismutase (SOD3) activity, protein, metabolites of the nitric oxide/cyclic GMP pathway, heme oxygenase-I and inducible nitric oxide synthase expression in aqueous humor or/and peripheral blood from fifty-six patients with retinitis pigmentosa and sixty subjects without systemic or ocular oxidative stress-related disease. Multivariate analysis of covariance revealed that retinitis pigmentosa alters ocular antioxidant defence machinery and the redox status in blood. Patients with retinitis pigmentosa present low total antioxidant capacity including reduced SOD3 activity and protein concentration in aqueous humor. Patients also show reduced SOD3 activity, increased TBARS formation and upregulation of the nitric oxide/cyclic GMP pathway in peripheral blood. Together these findings confirmed the hypothesis that patients with retinitis pigmentosa present reduced ocular antioxidant status. Moreover, these patients show changes in some oxidative-nitrosative markers in the peripheral blood. Further studies are needed to clarify the relationship between these peripheral markers and retinitis pigmentosa.

Rapid regression of exudative maculopathy in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome after intravitreal ranibizumab.

The idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome is a rare retinal vascular disorder characterized by multiple leaking aneurysmal dilations, retinal vasculitis, neuroretinitis and peripheral vascular ischemia. Visual loss mainly occurs due to the development of retinal neovascularization and/or exudative maculopathy. Although the treatment of choice has not yet been established, retinal photocoagulation seems to be the best option to control the disease and to prevent its progression. Herein, we report a case of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome with both retinal neovascularization and macular exudation successfully managed with intravitreal ranibizumab (Lucentis(®)) as adjunctive therapy to retinal photocoagulation.

Safety and Efficacy of Ranibizumab in Macular Edema following Retinal Vein Occlusion.

Macular edema is the leading cause of visual impairment in patients with retinal vein occlusion. Limited improvements may be obtained with laser photocoagulation or intravitreal triamcinolone. However, according to the data provided by randomized clinical trials, intravitreal injections of ranibizumab (Lucentis; Genentech, South San Francisco, CA) constitute a new effective and safe option for the management of these vision-threatening diseases. The aim of the present review is to summarize the clinical evidence of ranibizumab for macular edema due to retinal vein occlusions.

Adult coats' disease successfully managed with the dexamethasone intravitreal implant (ozurdex®) combined with retinal photocoagulation.

PURPOSE: To report a case of Coats' disease managed with the dexamethasone intravitreal implant Ozurdex(®) (Allergan, Inc., Irvine, Calif., USA) combined with retinal photocoagulation. METHODS: A 46-year-old female with 20/200 visual acuity was diagnosed with Coats' disease with secondary retinal vasoproliferative tumor. An initial approach was performed with an intravitreal injection of the sustained-release dexamethasone implant Ozurdex. After reattachment of the retina, the telangiectatic vessels were treated with laser photocoagulation. RESULTS: The patient's visual acuity improved to 20/25 after the intravitreal Ozurdex. No further recurrences of exudation were evident through the 12-month follow-up. CONCLUSIONS: Ozurdex may be an effective initial therapeutic approach for Coats' disease with immediate anatomical response and visual improvement.